Complejo de deformidades amnióticas, adhesiones, mutilación: interminable debate

Abstract

Amniotic deformities, adhesions, mutilations (ADAM) complex is a broad heterogeneous spectrum of congenital anomalies. ADAM complex is characterized by constriction rings, amputation of fingers or limbs and the presence of the amniotic band. However, it may also involve craniofacial disruptions, body wall defects and internal organ abnormalities. The aim of this review is to present the results found in regard to ADAM complex from its historical background, clinical manifestations, epidemiology, etc. In particular, our attention was focused on demonstrating the varying etiopathogenesis theories of ADAM complex and their contradictions. The study was conducted using the databases of PubMed, EBSCO host, Ovid, SpringerLink, Scopus, nature.com, JAMA and ScienceDirect with the following keywords for the search: "amniotic band syndrome", "amniotic band sequence", "Streeter dysplasia", "ADAM complex". In this study we used 22 full-text articles. Patients with ADAM complex require a complete pre- and postnatal evaluation to integrate the diagnosis and to decide on timely treatment. It is important for clinicians and surgeons to possess knowledge of this entity. Further research is necessary to establish a nosological basis.

Amniotic band syndrome associated with exencephaly: A case report and literature review

“…Its etiology is unknown. Several theories have been suggested to explain its etiopathogenesis, and the most accepted ones are as follows: (1) the intrinsic theory proposed by Streeter in 1930, which establishes that congenital constriction is a germplasm defect with vascular disruption and change of morphogenesis during gastrulation, forming the fibrous bands and causing limb necrosis[139] and (2) the extrinsic theory proposed by Torpin in 1965, which associated the defects of the extremities or limb amputations with amniotic rupture and compression of the bands. [3]…”

“…Van Allen et al , in 1987, also contributed with the theory of vascular disruption, preceded by Kino who conducted experiments in animal models in 1975. [3]…”

“…In particular, craniofacial abnormalities were classified as follows: (1) exencephaly/encephalocele with amniotic connections and without facial cleft; (2) similar cranial lesions, with facial clefts and with or without amniotic bands; (3) abdominoschisis and thoracoabdominoschisis; and (4) limb abnormalities. [3]…”

“…The diagnosis of ABS is difficult and occurs in only 29%–50% of cases. [3] The craniofacial deformities are often bizarre, and the internal organ abnormalities are rare. [2]…”

“…The first description of its etiopathogenesis was proposed by Montgomery in 1832. [3] Although studies by Orioli et al [4] showed that the extremities are more affected by this disease, Guzmán-Huerta et al [5] reported craniofacial abnormalities in 78% of the cases and defects of the extremities in 70%. Cranial changes range from encephalocele to acrania.…”